Abstract

The most common form of neurological injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial (SIT Trial), we sought to identify risk factors associated with SCI. In this cross-sectional study, we evaluated the clinical history and baseline laboratory values and performed magnetic resonance imaging of the brain in participants with SCA (HbSS or HbSβ° thalassemia) between the ages of 5 and 15 years with no history of overt stroke or seizures. Neuroradiology and neurology committees adjudicated the presence of SCI. SCI were diagnosed in 30.8% (251 of 814) participants who completed all evaluations, and had valid data on all pre-specified demographic and clinical covariates. The mean age of the participants was 9.1 years, with 413 males (50.7%). In a multivariable logistic regression analysis, lower baseline hemoglobin concentration p < 0.001; higher baseline systolic blood pressure (SBP), p = 0.013; and male gender, p = .026; were statistically significantly associated with an increased risk of a SCI. Hemoglobin concentration and SBP are risk factors for SCI in children with SCA and may be therapeutic targets for decreasing the risk of SCI. This study is registered at www.clinicaltrials.gov (NCT00072761).